Abstract
PurposePolyglandular autoimmune syndromes (PAS) are complex, heterogeneous disorders in which various autoimmune diseases can occur, affecting both endocrine and non-endocrine organs. In this meta-analysis, the prevalence of associated autoimmune disorders was investigated in PAS II and III.MethodsA comprehensive search in MEDLINE and Embase databases identified 479 studies with the keywords of PAS II and PAS III. 18 records containing a total of 1312 patients fulfilled our inclusion criteria (original studies reporting at least 10 cases and containing the combination of other autoimmune disorders) and were selected for further analysis. A meta-analysis of prevalence was performed using the random-effects model with the calculation of 95% confidence intervals (CI). Results of each meta-analysis were displayed graphically using forest plots.ResultsDistinction between PAS II and PAS III was made in 842 cases, of which 177 and 665 were PAS II and III (21.1 vs 78.9%), respectively. The prevalence of Hashimoto’s thyroiditis was significantly higher than that of Graves’s disease (39% [95% CI 17–65%] vs. 4% [95% CI 0–10%], respectively; p = 0.001). In PAS II, Addison’s disease (AD) coexisted with AITDs, T1DM or the combination of these conditions in 65, 18 and 10% of cases, respectively. In addition, one other endocrine and five non-endocrine organ-specific autoimmune disorders were reported. In PAS III, two other autoimmune endocrinopathies, six non-endocrine organ-specific, and four systemic autoimmune disorders were found in combination with AITDs.ConclusionsAITDs, T1DM and AD are the most common combinations in PAS, thus screening for these conditions seems to be reasonable.
Highlights
Autoimmune polyglandular syndromes (PAS) are complex, heterogeneous disorders in which various autoimmune diseases can occur, affecting both endocrine and non-endocrine organs
PAS II is defined by the presence of Addison’s disease (AD) and autoimmune thyroid diseases (AITDs) and/or type 1 diabetes mellitus (T1DM) [2]
A standardized form of numeric data was independently extracted by two investigators and manually populated into a purpose-designed Excel 2016 sheet (Office 365, Microsoft, Redmond, WA, USA) containing the following information: year of publication, type of study, participating centers, number of patients included and diagnosed with PAS, average age of the patients, sex, main disorders (AD, AITDs, T1DM, premature ovarian failure (POF), vitiligo, alopecia, coeliac disease, autoimmune hepatitis, pernicious anemia, autoimmune bowel disease, haemolytic anemia, systemic lupus erythematosus (SLE), psoriasis, Sjögren’s syndrome, rheumatoid arthritis (RA), myasthenia gravis, multiple sclerosis, hypoparathyroidism, hypophysitis) and their combinations
Summary
Autoimmune polyglandular syndromes (PAS) are complex, heterogeneous disorders in which various autoimmune diseases can occur, affecting both endocrine and non-endocrine organs. Certain autoimmune disorders such as T1DM and juvenile idiopathic arthritis (JIA) often present in childhood, whereas co-associated AITD might appear later during adulthood, altering both the initial diagnosis and the prevalence of PAS in childhood [14] The aims of this meta-analysis were to collect studies systematically to characterize the prevalence of autoimmune diseases other than the obligatory manifestations of PAS II and PAS III and to investigate the characteristics of PAS II and PAS III in relation to gender and age. A standardized form of numeric data was independently extracted by two investigators and manually populated into a purpose-designed Excel 2016 sheet (Office 365, Microsoft, Redmond, WA, USA) containing the following information: year of publication, type of study, participating centers, number of patients included and diagnosed with PAS, average age of the patients, sex, main disorders (AD, AITDs, T1DM, POF, vitiligo, alopecia, coeliac disease, autoimmune hepatitis, pernicious anemia, autoimmune bowel disease, haemolytic anemia, SLE, psoriasis, Sjögren’s syndrome, RA, myasthenia gravis, multiple sclerosis, hypoparathyroidism, hypophysitis) and their combinations. The authors of the original studies were contacted to obtain missing information
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